成人朗格汉斯细胞组织细胞增生症3例并文献复习

杨雪良; 王志红; 曹永彬; 徐丽昕; 李晓红; 吴亚妹; 闫蓓; 刘蓓; 刘周阳; 吴晓雄

doi:CNKI:11-3275/R.20111020.1444.002

成人朗格汉斯细胞组织细胞增生症3例并文献复习

Adult Langerhans cell histiocytosis: A report of 3 cases and literature review

摘要

摘要: 目的探讨成人朗格汉斯细胞组织细胞增生症(LCH)的临床表现、病理特点、治疗和预后。方法回顾分析3例成人朗格汉斯细胞组织细胞增生症并复习相关文献。结果 3例LCH患者分别为39岁女性、36岁男性、56岁女性,临床受累脏器包括耻骨(1/3)、髂骨(1/3)、额骨(1/3)、股骨(1/3)、肋骨(1/3)、皮肤(1/3)、淋巴结(1/3),免疫组化为CDla(+)、S-100(+),经联合化疗后,2例患者获得完全缓解,另1例多系统LCH患者反应较差,仍在进一步治疗观察中。结论成人LCH少见,临床表现多样,易误诊、漏诊,治疗需个体化。

Abstract: Objective To study the clinical manifestations,pathologic characteristics,treatment and prognosis of adult Langerhans cell histiocytosis（LCH）. Methods Three adult LCH patients were retrospectively analyzed with related literature reviewed. Results The 3 LCH patients were a female at the age of 39 years,a male at the age of 36 years,and a female at the age of 56 years with their pubis（1/3）,iliac（1/3）,frontal bone（1/3）,thigh bone（1/3）,ribs（1/3）,skin（1/3）,lymph nodes（1/3） involved.Immunohistochemistry was positive for CD1a and S-100.After combined chemotherapy,2 patients achieved complete remission and the other patient with multiple systems involved had a poor response and needed further treatment and observation. Conclusion Adult LCH is a rare disease with various clinical manifestations.It is easy to be misdiagnosed and missed,and should be treated according to the condition of each patient.

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